The full content of Annals is available to subscribers

Subscribe/Learn More  >
Reviews |

Ventricular Septal Defects in Adults

Naser M. Ammash, MD; and Carole A. Warnes, MD
[+] Article, Author, and Disclosure Information

From the Mayo Clinic and Mayo Foundation, Rochester, Minnesota.

Acknowledgment: The authors thank Becky Hendrickson for technical support.

Requests for Single Reprints: Naser M. Ammash, MD, Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.

Current Author Addresses: Drs. Ammash and Warnes: Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.

Ann Intern Med. 2001;135(9):812-824. doi:10.7326/0003-4819-135-9-200111060-00011
Text Size: A A A

Because congenital ventricular septal defects are of different sizes and locations, their clinical presentation, natural history, and treatment vary greatly. This review discusses the different types of ventricular septal defects commonly seen in adults in the authors' experience and in published literature. Ventricular septal defects are either isolated small defects or larger defects associated with pulmonary stenosis, pulmonary hypertension, or aortic regurgitation. These associations play an important role in the pathophysiologic consequences of the defect, its long-term complications, and treatment options. Knowledge of the different clinical presentations in adulthood and the specific features pertinent to these defects will help in the assessment and the care of adult patients with one of the most common congenital cardiac malformations.


Grahic Jump Location
Figure 1.
Septal defects.

Top. Positions of different ventricular septal defects. 1 = membranous; 2 = subarterial or supracristal; 3 = muscular or trabecular; 4 = inlet or canal. (Modified from Capelli and colleagues [14] with permission of Excerpta Medica.) Bottom left. Membranous ventricular septal defect (VSD), as seen from the left ventricle, partially obliterated by the septal leaflet of the tricuspid valve (SLTV). Bottom right. Muscular VSD as seen from the left ventricle. (Photographs courtesy of Dr. William D. Edwards, Division of Anatomic Pathology, Mayo Clinic Rochester.)

Grahic Jump Location
Grahic Jump Location
Figure 2.
Pathophysiology of aortic regurgitation.

In early systole (left), ejected blood from the left ventricle (LV) will be shunted through the ventricular septal defect. As a result, the anatomically unsupported coronary cusp and aortic sinus are driven into the right ventricle (RV) (middle); this is known as the Venturi effect. In diastole (right), the intra-aortic pressure forces the aortic valve leaflet to close, but the unsupported cusp (right or noncoronary) is pushed down into the left ventricular outflow tract away from the opposed coronary cusp, resulting in regurgitation. AR = aortic regurgitation; IVS = interventricular septum; PA = pulmonary artery. (Reproduced from Tatsuno and colleagues [23] with permission of the American Heart Association.)

Grahic Jump Location
Grahic Jump Location
Figure 3.
The cardiac examination in ventricular septal defect (VSD).

Top left. Holosystolic murmur of VSD. Top middle. Shortened systolic murmur of muscular VSD. Top right. Typical murmur of VSD with mild pulmonary stenosis (PS) showing the delayed pulmonary closure sound (P2). Bottom left. Systolic ejection murmur of severe pulmonary stenosis with delayed and reduced P2. Bottom middle. Eisenmenger complex with absence of the holosystolic murmur of VSD, a loud P2 secondary to pulmonary hypertension, and pulmonary regurgitation (PR) diastolic murmur. Bottom right. VSD murmur followed by diastolic murmur of aortic regurgitation (AR); A2 = aortic closure sound; C = ejection click; S1 = first heart sound.

Grahic Jump Location
Grahic Jump Location
Figure 4.
Electrocardiogram of a 42-year-old woman with Eisenmenger complex, demonstrating atrial fibrillation with right axis deviation, right ventricular hypertrophy, right bundle-branch block, and premature ventricular beat.
Grahic Jump Location
Grahic Jump Location
Figure 5.
Chest radiograph in a patient with the Eisenmenger complex, showing cardiomegaly with severe enlargement of the proximal pulmonary arteries and pruning of the outer pulmonary vessels.
Grahic Jump Location
Grahic Jump Location
Figure 6.
Echocardiographic examination of ventricular septal defects (VSDs).

Top left. Standard parasternal long-axis echocardiographic views show the membranous septum (MS), the infundibular septum (IS), and the trabecular muscular septum (TS). (Reproduced from Hagler and colleagues [13] with permission of the Mayo Foundation). Top right. Color-flow Doppler echocardiogram demonstrating a membranous VSD with a left-to-right shunt (red flow from left ventricle to right ventricle). Bottom left. Parasternal short-axis view. The asterisk indicates supracristal or subarterial ventricular septal defect in the right ventricular outflow tract. In the same basal view, the † indicates membranous ventricular septal defect in proximity to the tricuspid valve. Bottom right. Freeze-frame image of a membranous VSD (arrow), as seen on parasternal short-axis view. AO = aorta; AVS = atrioventricular septum; L = left coronary cusp; LA = left atrium; P = posterior noncoronary cusp; PA = pulmonary artery; R = right coronary cusp; RA = right atrium; RV = right ventricle.

Grahic Jump Location
Grahic Jump Location
Figure 7.
Kaplan–Meier survival curve of all patients with ventricular septal defects (n= 1252) from the Natural History Study (dashed line) compared with the expected survival curve (solid line) for a sex- and age-matched population.

The number in parentheses indicates the number of patients remaining under observation 25 years after admission. (Reproduced from Kidd and colleagues [28] with permission of the American Heart Association.)

Grahic Jump Location
Grahic Jump Location
Figure 8.
Survival rate for patients with ventricular septal defects by pulmonary artery systolic pressure.

The solid line indicates a pressure less than 50 mm Hg (n = 36); the dashed line indicates a pressure of 50 mm Hg or greater (n = 17). (Reproduced from Ellis and colleagues [25] with permission of the CV Mosby Company.)

Grahic Jump Location




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).


Submit a Comment/Letter
Submit a Comment/Letter

Summary for Patients

Clinical Slide Sets

Terms of Use

The In the Clinic® slide sets are owned and copyrighted by the American College of Physicians (ACP). All text, graphics, trademarks, and other intellectual property incorporated into the slide sets remain the sole and exclusive property of the ACP. The slide sets may be used only by the person who downloads or purchases them and only for the purpose of presenting them during not-for-profit educational activities. Users may incorporate the entire slide set or selected individual slides into their own teaching presentations but may not alter the content of the slides in any way or remove the ACP copyright notice. Users may make print copies for use as hand-outs for the audience the user is personally addressing but may not otherwise reproduce or distribute the slides by any means or media, including but not limited to sending them as e-mail attachments, posting them on Internet or Intranet sites, publishing them in meeting proceedings, or making them available for sale or distribution in any unauthorized form, without the express written permission of the ACP. Unauthorized use of the In the Clinic slide sets will constitute copyright infringement.


Buy Now for $32.00

to gain full access to the content and tools.

Want to Subscribe?

Learn more about subscription options

Related Articles
Topic Collections
PubMed Articles
Forgot your password?
Enter your username and email address. We'll send you a reminder to the email address on record.